Case Of Cutaneous T-cell Lymphoma Research Articles

Risk of Cutaneous TCell Lymphoma with Psoriasis Biologic Therapies.

The risk of developing cutaneous Tcell lymphoma (CTCL) in patients using psoriasis biologics has not been well characterized. The goals of this review were to investigate the incidence of CTCL in patients with psoriasis receiving biologic therapy in clinical trials and psoriasis registries, and to review cases of CTCL and biologic use reported in scientific publications. The US National Library of Medicine clinical trials database (clinicaltrials.gov) was queried to identify phase3 and 4 clinical trials of the 12 biologic agents currently FDA approved for psoriatic disease. The incidence of CTCL in these trials was examined and summarized. To examine the incidence of CTCL in psoriasis registries, a Medline search was conducted. Finally, we performed a systematic review of CTCL cases reported in the literature. Only two cases of CTCL were reported in 35,801 subjects with psoriasis receiving a biologic agent in the active arm of 108 psoriasis phase3 clinical trials. One of these CTCL cases was determined by the investigator to be CTCL misdiagnosed as psoriasis prior to randomization. No cases of CTCL were reported in 5440subjects with psoriasis in 34 phase4 clinical trials. Only one case of CTCL was identified in 34,111 registry subjects. In the literature, tumor necrosis factor (TNF) inhibitors had the highest number of reported cases of CTCL (34 cases), followed by interleukin (IL)-17 inhibitors (7 cases), and IL-12/23 inhibitors (6 cases). No cases of CTCL were found to be reported with IL-23 inhibitors. Our findings indicate that the development of CTCL is rare in the setting of psoriasis biologic use. Of the limited number of cases of CTCL found, most were in the setting of TNF inhibitor use and no cases of CTCL were reported in the setting of IL-23 inhibitor use.

A Rare and Fatal Case of Cutaneous T-Cell Lymphoma (CTCL)

Introduction: Cutaneous T-Cell Lymphoma (CTCL) is a form of Non-Hodgkin Lymphoma involving T-cell neoplasms mainly concentrated in the skin. Primary Cutaneous Peripheral T-Cell Lymphoma is Not Otherwise Specified (PTL-NOS) is the rarest case of CTCL disease.Case Presentation: A female, 35 years old, complained of a lump on her face. In the last 3 months, the lump felt soft, contained a liquid that, when it ruptures, secretes Blood, and the crusty scar will blacken and easily bleed with pain. Physical examination of pale conjunctiva on the face, neck, chest, abdomen, back, and legs obtained nodules with a chewy consistency with erythema appearance in varying size. Some nodules that appear were covered in blackish crustae. The right lung has a decrease in the sound of breath, accompanied by swelling in both limbs. The conclusion of bilateral femur skin biopsy impresses cutaneous T lymphoma cells. Within three months, cancer developed into lesions spread almost throughout the body, and due to the rapid and progressive nature of cancer, its diagnosis developed into PTL-NOS.Conclusions: Patients with PTL-NOS may come with solitary nodules such as red tumors in any area of the body. However, most often, patients come with symptoms of multifocal or diffuse scattered nodules. In enforcing the diagnosis, it takes a high level of suspicion, and multiple rebiopsies are necessary to enforce the diagnosis of CTCL.

A case of cutaneous T-cell lymphoma (CD − 8+) with multiple lesions treated with definitive radiotherapy

Cutaneous T-cell lymphoma (CTCL) is the most common primary cutaneous lymphoma but rarely encountered. It is often misdiagnosed with allergic or infectious dermatological conditions and is usually a diagnosis of exclusion. The subsets of CTCL primarily consist of CD4 + and CD30 + lymphoproliferative disorders including mycoses fungoides. Herein, we report a case of CD 8 + ve CTCL stage IA that was previously misdiagnosed with cutaneous tuberculosis with no response to antitubercular therapy. After a confirmatory diagnosis of CTCL with multiple sites involved, he was treated with definitive local radiotherapy alone and had a complete response to treatment with no recurrence at 5 year's follow-up.

Mycosis fongoïde après transplantation cardiaque

Skin cancer occurs frequently in organ transplant patients as a result of induced immunosuppression. Most cases involve carcinomas or B-cell lymphomas induced by the Epstein Barr virus (EBV). Cutaneous T-cell lymphomas remain rare. We report a case of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant recipient. A 68-year-old man who had received a heart transplant 21years earlier and was being treated with tacrolimus, mycophenolate mofetil and prednisolone had been presenting a psoriasiform rash on his trunk, limbs and head for 4years. The rash was resistant to both PUVA therapy and topical corticosteroids. Histopathological examination suggested epidermotropic cutaneous T-cell lymphoma. There was no impairment of the patient's general state of health nor any adenopathy. Molecular biology revealed TCR rearrangement in both blood and skin. Screening for circulating Sézary cells was negative, and PET scan revealed no signs of extracutaneous localization. Mechlorethamine showed little efficacy, bexarotene was complicated by dysthyroidism, hypertriglyceridemia was ineffective, methotrexate was contraindicated because of calcineurin inhibitor-related chronic kidney failure, and interferon could not be given due to the context of heart transplantation. Finally, we treated our patient with gemcitabine, which initially proved effective but was later complicated by septic shock that resulted in the patient's death. The particularities of our observation are the onset of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant patient, and the therapeutic difficulties encountered in a setting of transplantation with immunodepression.

A Case of Cutaneous T-Cell Lymphoma with Nodular Skin Lesions, Third Nerve Palsy and Peripheral Vascular Disease: Can It Be a Case of Angiocentric Lymphoma?

Angiocentric lymphoma is a subtype of cutaneous T-cell lymphoma, characterized by occlusion of vessels by lymphoid infiltrate. Here, we report a case of biopsy confirmed cutaneous T-cell lymphoma who presented with third cranial nerve palsy, peripheral vascular disease and nodular skin lesions in the right thigh. All these findings of this case can be explained by mechanism of vascular infiltration and tissue destruction in angiocentric lymphoma which is a rare disorder.Birdem Med J 2016; 6(1): 40-42

Adaptation of the Stanford technique for treatment of bulky cutaneous T-cell lymphoma of the head

Electron beam radiation therapy is an effective treatment for cutaneous T-cell lymphoma (CTCL).1,2 The first description of total skin electron therapy came from Stanford University.1,3 Prolonged treatment to ≥3000 cGy in 6 to 7 weeks is not feasible for many patients in a palliative setting. Hypofractionated regimens are associated with high response rates.4-8 We describe a case of bulky CTCL of the head treated with a unique adaptation of the Stanford technique.

Primary cutaneous follicular helper T-cell lymphoma.

Follicular helper T-cells (TFH) represent a specific subset of CD4-positive helper T-cells that help B-cells to differentiate into long-lived antibody-secreting plasma cells or memory B-cells. The expression of TFH markers in neoplastic T-cells, traditionally related to the angioimmunoblastic (AITL) subgroup of peripheral T-cell lymphomas, is nowadays well-known to be more widespread than previously thought. We report hereby a case of cutaneous T-cell lymphoma in a 75-year-old woman, whose morphological and immunophenotypical features raises the differential diagnosis between cutaneous involvement by AITL and the recently described primary cutaneous T-cell lymphoma with follicular helper-phenotype.

Development of mycosis fungoides after bone marrow transplantation for chronic myeloid leukaemia: transmission from an allogeneic donor

We report on a patient who developed donor-derived cutaneous T-cell lymphoma (CTCL) 4 years after successful treatment of chronic myeloid leukaemia with an allogeneic bone marrow transplant. The patient developed an eczematous rash unresponsive to topical therapy and immunosuppression. When CTCL was diagnosed in the recipient, his sibling donor had been attending his local dermatology unit with a maculosquamous rash, which proved subsequently to be mycosis fungoides. An identical pattern of donor and recipient clonality assessment and T-cell receptor gene sequencing indicated that the CTCL was probably transmitted in the bone marrow harvest. This suggests that CTCL cells circulate in the marrow at an early subclinical stage in this disease. This is the second case of donor-derived CTCL reported to date.

Cutaneous T-Cell Lymphoma in Sub-Saharan Africa

The incidence and economic burden of cancer in sub-Saharan Africa is increasing, and innovative strategies are needed to improve prevention and care in this population. This article uses a case of cutaneous T-cell lymphoma in Uganda to propose guidelines for the diagnosis and treatment of this disease in resource-limited settings. These guidelines were developed from the consensus opinion of specialists at the Uganda Cancer Institute and Fred Hutchinson Cancer Research Center as part of an established collaboration. Areas for future investigation that can improve the care of patients in this region are identified.

Risk of Second Primary Malignancies (SPMs) Following Bortezomib (Btz)-Based Therapy: Analysis of Four Phase 3 Randomized Controlled Trials in Previously Untreated or Relapsed Multiple Myeloma (MM)

Abstract 2933 Background:MM patients have a significantly increased risk of developing certain SPMs subsequent to their initial diagnosis, including a 3.49-fold increased risk of leukemia and, specifically, an 8.32-fold increased risk of acute non-lymphocytic leukemia (but no overall increased risk of solid tumor SPMs; Surveillance, Epidemiology and End Results [SEER] data 1973–2000). The relative SPM risk increases with age and time after initial diagnosis; the risk of leukemia rises from a 1.22-fold increase within 1 year after diagnosis to 3.12-fold, 7.01-fold, and 5.45-fold increases at 1–4, 5–9, and ≥10 years, respectively. An elevated risk of SPMs may be particularly associated with the use of specific therapeutic agents, including conventional or high-dose cytotoxic chemotherapy. Here we report an analysis of data from four phase 3, randomized, controlled trials of Btz alone or in combination to determine whether Btz treatment is associated with an increased SPM risk. Methods:Data were reviewed from: 1) the APEX study of Btz versus high-dose dexamethasone (Dex), and 2) the MMY-3001 study of Btz plus pegylated liposomal doxorubicin (PLD) versus Btz in patients with relapsed or refractory MM after 1–3 prior therapies; 3) the VISTA study of Btz plus melphalan-prednisone (VMP) versus MP in previously untreated transplant-ineligible patients; and 4) the HOVON65/GMMG-HD4 study of Btz, doxorubicin, and Dex (PAD) induction plus Btz maintenance post-transplant versus vincristine, doxorubicin, and Dex (VAD) induction plus thalidomide (Thal) maintenance in previously untreated transplant-eligible patients. Planned duration of Btz therapy was 39 weeks in APEX, 24 weeks in MMY-3001, 54 weeks in VISTA, and 9 weeks induction plus 2 years of maintenance in HOVON65/GMMG-HD4. For APEX, MMY-3001, and VISTA, clinical trial databases were reviewed for events within the MedDRA system organ class of ‘neoplasms', and new malignancies developing during or after treatment were recorded (excluding non-melanomatous skin cancers and in situ malignancies). In addition, for VISTA, data were obtained from an SPM survey after a median follow-up of 5 years. For HOVON65/GMMG-HD4, data were prospectively collected; median follow-up was 42 months. The incidence rate (IR) of SPMs was expressed as the number per 100 patient-years (pt-yrs). Results:The risk of SPMs with Btz-based therapy appeared uniformly low across all four phase 3 studies in different MM patient populations (Table). A total of 25 SPMs were seen in 1718 Btz-treated patients, including three cases of acute myeloid leukemia/myelodysplastic syndromes, one B-cell malignancy, and one case of cutaneous T-cell lymphoma (in a patient with substantial prior alkylating agent exposure for treatment of MM), plus 20 reports of solid tumors (reflecting the higher overall incidence of these tumors). The IR ranged from 0 in the single-agent Btz arm of MMY-3001 to 1.66 with VMP in VISTA, with an IR of 0.88 in APEX and 0.3 in the PAD arm of HOVON65/GMMG-HD4; median age of Btz-treated patients was highest in VISTA, at 71 years. Overall rates (%) also appeared higher in VISTA, probably due to the longer follow-up and older population, plus the potential effects of concomitant chemotherapy. In the three studies with non-Btz control arms, the IR did not appear to be increased with Btz-based therapy versus the control arm, and across all Btz-containing arms in the four studies, the IRs for hematologic malignancies and solid tumors were consistent with SEER estimates (2004–2008 data) of the overall incidence of malignancies in the US population, of 1.1, 1.9, and 2.4 per 100 pt-yrs in individuals aged 55–64, 65–74, and 75–84 years, respectively. Conclusions:Btz-based therapy for MM does not appear to be associated with an increased risk of either hematologic or solid tumor SPMs, with IRs consistent with SEER data for IRs in the overall US population.SPMs in phase 3 studies of Btz-based therapyAPEXMMY-3001VISTAHOVON65/ GMMG-HD4ArmBtzDexBtz+PLDBtzVMPMPPAD/BtzVAD/ThalN331332318316340337413414Median age, yrs62616162717157*57*SPMs, n (%)1 (0.30)01 (0.31)019 (5.6)13 (3.9)4 (0.97)11 (2.7)Heme1 (0.30)0003 (0.88)3 (0.89)1 (0.24)4 (0.97)Solid001 (0.31)016 (4.71)10 (2.97)3 (0.73)7 (1.69)Pt-yrs113.994.2106.3104.51167100412391183IR0.8800.9401.661.300.30.9Heme0.880000.260.300.0750.33Solid000.9401.401.000.2250.57Heme, hematologic malignancies.*ASH 2010 data Disclosures:San Miguel:Janssen-Cilag: Consultancy; Celgene: Consultancy; Millennium Pharmaceuticals, Inc: Consultancy. Richardson:Celgene: Consultancy; Janssen Research & Development: Consultancy; Millennium Pharmaceuticals, Inc.: Consultancy. Orlowski:Millennium Pharmaceuticals, Inc.: Consultancy, Research Funding; Novartis: Consultancy; Onyx: Consultancy; Cephalon: Consultancy; Centocor: Consultancy; Celgene: Consultancy, Research Funding. Goldschmidt:Janssen-Cilag: Consultancy; Celgene: Consultancy; Millennium Pharmaceuticals, Inc.: Consultancy. Corzo:Millennium Pharmaceuticals, Inc.: Employment. Satler:Millennium Pharmaceuticals, Inc.: Employment. Esseltine:Millennium Pharmaceuticals, Inc.: Employment, Equity Ownership; Johnson & Johnson: Equity Ownership. Neuwirth:Millennium Pharmaceuticals, Inc.: Employment. Ponsillo:Millennium Pharmaceuticals, Inc.: Employment. Cakana:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership. King:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership. Deraedt:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership. Desai:Janssen Global Services: Employment; Johnson & Johnson: Equity Ownership. Lutska:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership. Gifkins:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership. Liu:Janssen Research & Development: Employment; Johnson & Johnson: Equity Ownership.

A Case of Cutaneous T-Cell Lymphoma in a 14-year-old Male.

A Case of Cutaneous T-Cell Lymphoma in a 14-year-old Male.

Mycosis fungoides/Sézary syndrome: report of an unusual case

Sézary syndrome (SS) is an uncommon form of cutaneous T cell lymphoma (CTCL) with a classical triad of lymphadenopathy, characteristic circulating lymphoma cells (Sézary cells) and erythrodermatous skin involvement with classical mycosis fungoides (MF)-like histological picture. A 32-year-old woman presented with this classical triad; however, her skin involvement, histologically, was in the form of folliculotropic MF, rather than the usual classical form of MF. In the vast majority of cases, the cutaneous involvement in SS resembles conventional MF, histologically. One case of CD30-positive CTCL with pilotropic MF has been reported. However, English literature does not describe any case of SS with folliculotropic MF with typical immunophenotype of SS thus far. We presume that this case represents the first report of SS with folliculotropic MF histologically, displaying the typical CD30-negative immunophenotype.

Candidose cutanée diffuse révélatrice de lymphome T cutané : 2 cas

During the course of immunodeficiency diseases, severe candidiasis can occur with extensive cutaneous and mucous membrane lesions. However, blood dyscrasias are very rarely revealed by diffuse candidiasis. We report two case of cutaneous T-cell lymphoma revealed by extensive and atypical cutaneous candidiasis. Case No. 1:A 72-year-old woman presented a pruritic rash of circinate, serpiginous patches on glabrous skin and skinfolds with multiple intertrigo and rapidly worsening palmoplantar keratoderma. All mycological skin specimens tested positive for Candida albicans. Histological examination of a biopsy sample from a serpiginous patch revealed the presence of fungal elements while palmoplantar keratoderma biopsy showed an epidermotropic lymphocytic infiltrate in the superficial dermis evocative of mycosis fungoides. Blood tests showed a white cell count of 28 600/mm3 with 14% circulating Sezary cells and a T-cell clone. The T-cell lymphoma was treated with methotrexate, but the disease worsened a few months later, progressing to CD30- large T-cell pleomorphic lymphoma. The patient died of severe sepsis. Case No 2:A 60-year-old man presented a macular rash over the face, trunk and skinfolds as well as erythematous scaly annular plaques of the glabrous skin with lymphadenopathy. Cultures of skin scrapings were all positive for Candida albicans. Blood tests showed a white cell count of 15 000/mm3 with 30% circulating Sezary cells. A trunk patch biopsy revealed the histological appearance of mycosis fungoides. There was a T-cell clone in the peripheral blood and skin. In both cases, the patients presented with widespread annular and erythematous scaly lesions of the glabrous skin and skinfolds with evidence of Candida albicans on fungal tests of all skin scrapings. The discovery of circulating Sezary cells on a systematic smear for hyperleukocytosis led us to suspect underlying cutaneous T-cell lymphoma, which was confirmed by biopsy of the skin lesions accompanying the mycoses. Widespread cutaneous candidiasis can occur in patients with cell-mediated immunodepression. Cutaneous T-cell lymphoma can enhance such candidiasis through interference with skin integrity and impairment of cell-mediated immunity, with large amounts of IL10 and TGF-B, increased secretion of soluble interleukin-2 receptors (CD25) and impaired CD8 suppressor cell function.

Hypopigmented mycosis fungoides in a 10-year-old boy

Cutaneous T-cell lymphoma (CTCL) presenting with hypopigmented lesions is an uncommon clinical variant of the disease, usually described in dark-skinned patients. We report a case of hypopigmented CTCL in a 10-year-old boy. The disease has responded favorably to narrowband UVB therapy. This case illustrates the importance of clinical suspicion for mycosis fungoides in patients with widespread hypopigmentation.

Cutaneous T‐cell lymphoma in a 21‐year‐old male with Wolf–Hirschhorn syndrome

We describe a case of cutaneous T-cell lymphoma occurring in a 21-year-old male with Wolf-Hirschhorn syndrome (WHS) due to a chromosome 4p16.3 deletion. This is the first documented case report of malignancy occurring in an adult with WHS. We also review the literature regarding patients with WHS and the joint occurrence of malignancy and discuss genetic changes involving chromosome 4 which may have contributed to the genesis of our patient's lymphoma.

Gamma/Delta T-Cell Lymphoma of Duodenal BulbA Case Report

Gamma/delta T-cells represent a minor subset of T lymphocytes in the peripheral blood of limited diversity and a tissue restricted distribution. 1 Groh V Porcelli S Fabbi M Lanier LL Picker LJ Anderson T et al. Human lymphocytes bearing T cell receptor gamma/delta are phenotypically diverse and evenly distributed throughout the lymphoid system. J Exp Med. 1989; 169: 1277-1294 Crossref PubMed Scopus (437) Google Scholar , 2 Falini B Flenghi L Pileri S Pelicci P Fagioli M Martelli MF et al. Distribution of T cells bearing different forms of the T cell receptor gamma/delta in normal and pathological human tissues. J Immunol. 1989; 143: 2480-2488 PubMed Google Scholar These T-cells have a predilection for the red pulp of the spleen and the GI tract. 1 Groh V Porcelli S Fabbi M Lanier LL Picker LJ Anderson T et al. Human lymphocytes bearing T cell receptor gamma/delta are phenotypically diverse and evenly distributed throughout the lymphoid system. J Exp Med. 1989; 169: 1277-1294 Crossref PubMed Scopus (437) Google Scholar , 2 Falini B Flenghi L Pileri S Pelicci P Fagioli M Martelli MF et al. Distribution of T cells bearing different forms of the T cell receptor gamma/delta in normal and pathological human tissues. J Immunol. 1989; 143: 2480-2488 PubMed Google Scholar , 3 Bordessoule D Gaulard P Mason DY Preferential localisation of human lymphocytes bearing gamma delta T cell receptors to the red pulp of the spleen. J Clin Pathol. 1990; 43: 461-464 Crossref PubMed Scopus (72) Google Scholar Most neoplasms arising from the gamma/delta T-cell subpopulation are hepatosplenic gamma/delta T-cell lymphomas, a distinctive clinicopathologic entity. 4 Wong KF Chan JK Matutes E McCarthy K Ng CS Chan CH et al. Hepatosplenic gamma delta T-cell lymphoma. A distinctive aggressive lymphoma type. Am J Surg Pathol. 1995; 19: 718-726 Crossref PubMed Scopus (96) Google Scholar , 5 Cooke CB Krenacs L Stetler-Stevenson M Greiner TC Raffeld M Kingma DW et al. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood. 1996; 88: 4265-4274 PubMed Google Scholar Several cases have been reported of non-hepatosplenic gamma/delta T-cell lymphoma in specific sites such as cutaneous tissue, 6 Fujita M Miyachi Y Furukawa F Toichi E Furukawa I Nakajima N Imamura S A case of cutaneous T-cell lymphoma expressing gamma delta T-cell receptors. J Am Acad Dermatol. 1993; 28: 355-360 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 7 Arnulf B Copie-Bergman C Delfau-Larue MH Lavergne-Slove A Bosq J Wechsler J et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. 1998; 91: 1723-1731 PubMed Google Scholar the nasopharyngeal region, 7 Arnulf B Copie-Bergman C Delfau-Larue MH Lavergne-Slove A Bosq J Wechsler J et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. 1998; 91: 1723-1731 PubMed Google Scholar , 8 Kanavaros P Lescs MC Briere J Divine M Galateau F Joab I et al. Nasal T-cell lymphoma: a clinicopathologic entity associated with peculiar phenotype and with Epstein-Barr virus. Blood. 1993; 81: 2688-2695 PubMed Google Scholar the GI tract, 7 Arnulf B Copie-Bergman C Delfau-Larue MH Lavergne-Slove A Bosq J Wechsler J et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. 1998; 91: 1723-1731 PubMed Google Scholar , 9 Lavergne A Brocheriou I Delfau MH Copie-Bergman C Houdart R Gaulard PH Primary intestinal gamma-delta T-cell lymphoma with evidence of Epstein-Barr virus. Histopathology. 1998; 32: 271-276 Crossref PubMed Scopus (23) Google Scholar , 10 Tsujikawa T Itoh A Bamba M Andoh A Hodohara K Inoue H et al. Aggressive jejunal gamma delta T-cell lymphoma derived from intraepithelial lymphocytes: an autopsy case report. J Gastroenterol. 1998; 33: 280-284 Crossref PubMed Scopus (6) Google Scholar , 11 Katoh A Ohshima K Kanda M Haraoka S Sugihara M Suzumiya J et al. Gastrointestinal T cell lymphoma: predominant cytotoxic phenotypes, including alpha/beta, gamma/delta T cell and natural killer cells. Leuk Lymphoma. 2000; 39: 97-111 Crossref PubMed Scopus (57) Google Scholar and a single case involving the thyroid gland. 12 Yamaguchi M Ohno T Kita K gamma/delta T-cell lymphoma of the thyroid gland. N Engl J Med. 1997; 336: 1391-1392 Crossref PubMed Scopus (38) Google Scholar Reported here is the first case of gamma/delta T-cell lymphoma of the duodenal bulb. Expression of gamma/delta T-cell receptor (TCR) was confirmed by both genetic analysis of the TCR gamma and the delta chain by using the polymerase chain reaction (PCR) method, and immunostaining of TCR gamma. Systemic examination by CT and gallium scintigraphy incidentally revealed metastasis to the right lobe of the thyroid gland. The patient was treated with combination chemotherapy and was disease free at one year follow-up.

腫よう随伴性小脳変性症を認めた皮膚Ｔ細胞リンパ腫の１例

腫よう随伴性小脳変性症を認めた皮膚Ｔ細胞リンパ腫の１例

Unusual case of cutaneous T-cell lymphoma with a prominent reactive population of B lymphocytes.

Unusual case of cutaneous T-cell lymphoma with a prominent reactive population of B lymphocytes.

Initial Report of a Cutaneous T-Cell Lymphoma Appearing on the Auricular Helix

We report a case of cutaneous T-cell lymphoma in which the first sign of disease was involvement of the superior auricular helix. A review of the literature reveals that T-cell lymphoma often presents cutaneously, but it usually does not involve the ear, and an auricular lesion is rarely the first sign. The uncommon nature of this presentation, in addition to the potential need for multiple biopsies for a tissue diagnosis, can lead to a delay in diagnosis. When evaluating skin lesions in the head and neck, a high index of suspicion for cutaneous T-cell lymphoma is a key factor in its early diagnosis.

Lymphomatoid Papulosis Associated with Cutaneous T-Cell Signet Ring Lymphoma

The authors describe a new case of cutaneous T-cell lymphoma of signet ring cell type with CD-30 (Ki-t) expression. The patient was a 52-year-old woman with a 20-year history of recurrent papulonodular lesions characteristic of lymphomatoid papulosis (LP). Subsequent to developing the signet ring cell lymphoma (SRL) the patient's cutaneous lesions did not resolve as previously, and a skin biopsy showed a diffuse proliferation of atypical lymphoid cells with cytoplasmic vacuoles. SRL was first reported in 1985 by Weiss, and a literature search found only six further reports since then. Vaillant suggests that the peculiar morphology is retated to the special indolent course of this condition and that the cutaneous form should be considered a distinct entity. This is the first report of a patient with LP who subsequently developed a cutaneouis T-cell signet ring lymphoma with CD-30 antigen expression. The case could be an example of a so-called borderline lymphoma.